Abstract：
Kelchlike protein 11 antibodies (KLHL11-IgGs) were first described in 2019 as a marker of paraneoplastic neurological syndromes (PNSs). They have mostly been associated with testicular germ cell tumors (tGCTs).
Two patients with KLHL11-IgG encephalitis are reported, and the literature is comprehensively reviewed.
Patient 1 had been in remission from a tGCT 10 years prior. He developed episodic vertigo and diplopia progressing over a few days. Treatment with corticosteroids (CSs) was started a few days after symptom onset. Patient 2 had transient diplopia, which resolved spontaneously. Visual problems persisted for 7 months, when he additionally developed a progressive cerebellar syndrome. One year after onset, CS treatment was started. Initial magnetic resonance imaging was unremarkable in both patients, but analysis of cerebrospinal fluid (CSF) revealed chronic inflammation. KLHL11-IgG was positive in both patients (Patient 1 only in CSF, Patient 2 in serum). Neoplastic screening has so far not revealed any signs of active underlying malignancy. We found 15 publications of 112 patients in total with KLHL11-IgG encephalitis. Most patients (n = 82) had a cerebellar syndrome with or without signs of rhombencephalitis. The most common symptoms were ataxia (n = 82) and vertigo (n = 47), followed by oculomotor disturbances (n = 35) and hearing disorders (n = 31). Eighty of 84 patients had a GCT as an underlying tumor.
Our cases demonstrate classical symptoms of KLHL11-IgG encephalitis. Early diagnosis and therapy are imperative. As with other PNSs, clinical awareness is needed and further studies are required especially in regard to therapeutic management.
Two patients with KLHL11-IgG encephalitis are reported, and the literature is comprehensively reviewed.
Patient 1 had been in remission from a tGCT 10 years prior. He developed episodic vertigo and diplopia progressing over a few days. Treatment with corticosteroids (CSs) was started a few days after symptom onset. Patient 2 had transient diplopia, which resolved spontaneously. Visual problems persisted for 7 months, when he additionally developed a progressive cerebellar syndrome. One year after onset, CS treatment was started. Initial magnetic resonance imaging was unremarkable in both patients, but analysis of cerebrospinal fluid (CSF) revealed chronic inflammation. KLHL11-IgG was positive in both patients (Patient 1 only in CSF, Patient 2 in serum). Neoplastic screening has so far not revealed any signs of active underlying malignancy. We found 15 publications of 112 patients in total with KLHL11-IgG encephalitis. Most patients (n = 82) had a cerebellar syndrome with or without signs of rhombencephalitis. The most common symptoms were ataxia (n = 82) and vertigo (n = 47), followed by oculomotor disturbances (n = 35) and hearing disorders (n = 31). Eighty of 84 patients had a GCT as an underlying tumor.
Our cases demonstrate classical symptoms of KLHL11-IgG encephalitis. Early diagnosis and therapy are imperative. As with other PNSs, clinical awareness is needed and further studies are required especially in regard to therapeutic management.
摘要：
背景：Kelch样蛋白11抗体（KLHL11-IgG）于2019年首次被描述为副肿瘤神经综合征（PNS）的标志物。它们大多与睾丸生殖细胞肿瘤（tGCT）有关。
方法：报告2例KLHL11-IgG脑炎患者并综合文献复习。
结果：患者1在10年前已经从tGCT缓解。几天后，他出现了发作性眩晕和复视。症状发作后几天开始使用皮质类固醇（CS）治疗。患者2具有自发消退的短暂性复视。视力问题持续了7个月，当他还发展为进行性小脑综合征时。发病后1年,开始CS治疗。两名患者的初始MRI均无异常，但脑脊液（CSF）分析显示慢性炎症。两名患者的KLHL11-IgG均为阳性（患者1仅在CSF中，患者2的血清)。迄今为止，肿瘤筛查尚未发现任何活跃的潜在恶性肿瘤的迹象。我们共发现112例KLHL11-IgG脑炎患者中的15篇出版物。大多数患者（n=82）患有小脑综合征，有或没有菱形脑炎的迹象。最常见的症状是共济失调（n=82）和眩晕（n=47）。其次是动眼障碍（n=35）和听力障碍（n=31）。84例患者中有80例具有GCT作为潜在肿瘤。
结论：我们的病例表现出典型的KLHL11-IgG脑炎症状。早期诊断和治疗势在必行。与其他PNS一样，需要临床意识，需要进一步的研究，特别是在治疗管理方面。
方法：报告2例KLHL11-IgG脑炎患者并综合文献复习。
结果：患者1在10年前已经从tGCT缓解。
结论：我们的病例表现出典型的KLHL11-IgG脑炎症状。
